Medical and psychosocial outcome of children with congenital central hypoventilation syndrome.

摘要: We report the long-term medical and psychosocial outcome of 13 children with congenital central hypoventilation syndrome. One child (8%) died before initial hospital discharge. Of remaining 12 children, 11 (92%) have been successfully cared for in their natural or foster parents' homes. Home ventilatory support was provided positive-pressure ventilation, negative-pressure diaphragm pacers. After an lengthy hospitalization, spent little time hospital. Severe complications were uncommon but included cor pulmonale (one child), poor growth (two children), seizure disorder (three children). Most functioned slow-learner range mental processing, a composite score (Kaufman Assessment Battery Children) 78±20 (SD); two mentally retarded, one above normal range. The children's care givers assessed as having low levels psychologic distress (Symptom Checklist 90—Revised) good coping resources (Coping Resources Inventory) high level marital discord. able to attend school partake childhood activities. conclude that modern techniques home CCHS can outcome. speculate early diagnosis prevention intermittent hypoxia will improve physical